Presentation
The objective is to evaluate the present status and future role of free vascular fibular transfer in treating congenital pseudoarthrosis of the tibia. [ncbi.nlm.nih.gov]
Presenting problem: The table summarises patient characteristics, presenting problem, safeguarding assessment and action taken by social services. Café-au-lait lesions were present in all 3 patients. [bone-abstracts.org]
Patient with type 1 CPT usually presents early in life, before 2 years of age & have a good prognosis with treatment. [de.slideshare.net]
At the last follow-up, 14 patients presented with a mean of 12 mm (10–18) of FO. With the exception of one patient, all patients with FO presented with atrophic-type CPT. [bmcmusculoskeletdisord.biomedcentral.com]
Entire Body System
- Surgical Procedure
Numerous surgical procedures have been used including bone grafting, fixation, pulsed electromagnetic field, vascularized fibular bone grafting and lengthening devices. [ncbi.nlm.nih.gov]
Intraoperative fluoroscopy is useful for monitoring this procedure. Long-term bracing is mandatory during the growth years to minimize progressive valgus ankle deformity, or surgical treatment with the Langenskiöld procedure may be indicated. [orthopedicnotes.blogspot.com]
[…] of a healed, straight extremity by skeletal maturity, with as few surgical procedures as possible, is essential for a child with congenital pseudarthrosis of the tibia. [journals.lww.com]
Hence, patients may request amputation despite the fact that union has finally been gained.2'2 Early amputation as a definitive treatment may avoid numerous surgical procedures and a lifetime of disability from casts, braces, and other aspects of treatment [healio.com]
- Short Stature
stature ( Aarskog-Scott syndrome, Cockayne syndrome, Cornelia de Lange Syndrome, Dubowitz syndrome, Noonan syndrome, Robinow syndrome, Silver-Russell dwarfism, Seckel syndrome, Smith-Lemli-Opitz syndrome ) limbs ( Adducted thumb syndrome, Holt-Oram syndrome [wikidoc.org]
- Falling
The fractures were caused by an automobile-pedestrian accident (seven patients), an automobile-bicycle accident (four patients), fall from a height (two patients), and gunshot wound (one patient). [eoj.eg.net]
Musculoskeletal
- Fracture
The frequency of re-fracture after primary union varies from 14% to 60% Anatomic alignment of the tibia and fibula minimize the risk of re-fracture. [slideplayer.com]
An “ insufficiency” or “stress” fracture develops in the cortex of the tibia and gradually extends through the sclerotic bone. With completion of the fracture, healing fails to occur, and the fracture widens and becomes a pseudarthrosis. [orthopedicnotes.blogspot.com]
[…] minimize the risk of re-fracture. [doi.org]
Radiographs showed the fracture of the right lower tibia with bony sclerosis and a localized fusiform osteolytic lesion at the fracture site. The affected tibia bowed anteriorly and the medullary space in the lower tibia was narrow. [ncbi.nlm.nih.gov]
Treatment
Treatment of congenital pseudarthrosis of the tibia by the Ilizarov method. J Pediatr Orthop. 1990;10:786–90. [PubMed] [Google Scholar] 55. Grill F. Treatment of congenital pseudarthrosis of tibia with the circular frame technique. [ncbi.nlm.nih.gov]
and union after one additional treatment in 1 child. [42] Mean age at the time of treatment was 8 years. [doi.org]
- patellar tendon bearing - total contact orthosis, which is used to try to prevent frxs or to control developing ones; - Surgical Treatment Options: - once frx ensues, treatment includes open reduction, internal fixation, & bone grafting w/ autogenous [wheelessonline.com]
treatment with ilizarov frame seems to be the most effective form of treatment 24 [slideplayer.com]
Prognosis
The prognosis of CPT is very much related to the radiologic classification. Crawford type 2 and Crawford type 4 pseudoarthrosis have a worse prognosis, with a lower percentage of fusion at the site of pseudoarthrosis. [ncbi.nlm.nih.gov]
Type 2 have a relative poor prognosis. Type 3 cases are usually of late onset type which develops as a stress fracture of congenital dysplastic tibia or in a healed tibia after previous bone grafting & has the best prognosis. [de.slideshare.net]
Prognosis continues to be reserved especially in clepsydra forms and those localised to the lower third of the tibia. [moh-it.pure.elsevier.com]
The prognosis is good if the lesion is located only on the fibula, an extension to the tibia has a prognosis similar to type II, Type VI Associated with an intraosseous fibroma or a schwannoma. [boneandspine.com]
The prognosis depends on the aggressiveness and treatment of the intraosseous lesion. [orthopedicnotes.blogspot.com]
Etiology
Congenital pseudoarthrosis of the tibia (CPT) is an uncommon disease whose etiology and pathogenesis is unknown. [ncbi.nlm.nih.gov]
Pathology The etiology is unclear, however, around 50% of cases are associated with neurofibromatosis type 1 (NF1). Overall, 10% of patients with NF1 are diagnosed with tibial pseudoarthrosis. [radiopaedia.org]
Abstract The purpose of study was to refine frequency of etiological factors and characteristics of congenital pseudarthrosis of tibia (CPT). Materials and Methods. [journals.eco-vector.com]
Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data. J Pediatr Orthop B 2000; 9:11-15 [Google Scholar] 5. Grill F, Bollini G, Dungl P, et al. [ajronline.org]
The etiological nature of this lesion is unclear, however there is a strong association between CPT and neurofibromatosis type I (NF-I). [jlponline.org]
Epidemiology
Introduction Define/describe the condition Anatomy Describe the pertinent anatomy Pathogenesis Describe the biomechanics/biologic basis of the disorder or the mechanism of injury Natural History Describe the natural history, epidemiology and prognosis [orthopaedicsone.com]
Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data. J Pediatr Orthop B 2000; 9:11-15 [Google Scholar] 5. Grill F, Bollini G, Dungl P, et al. [ajronline.org]
Horn J, Steen H, Terjesen T (2013) Epidemiology and treatment outcome of congenital pseudarthrosis of the tibia. J Child Orthop 7(2):157–166 PubMedCentral CrossRef PubMed 20. [springermedizin.de]
Hefti, F, Bollini, G, Dungl, P, Fixsen, J, Grill, F, Ippolito, E, et al. 2000 Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data J Pediatr Orthop B 9 11 5 PubMed Google Scholar 16. [link.springer.com]
Congenital pseudoarthrosis of the tibia: History, etiology, classification and epidemiologic data. J Pediatr Orthop B 2000;9:11-5. [ PUBMED ] 2. Andersen KS, Bohr H, Sheppen O. Congenital angulation of the lower leg. Crus curvatum congenitum. [jlponline.org]
Pathophysiology
Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-64. Wang K, Allen L, Fung E, et al. Bone scintigraphy in common tumors with osteolytic components. Clin Nucl Med 2005;30:655-71. Tuncel M, Kiratli PO, Gedikoglu G. [journals.sfu.ca]
A relationship with type 1 neurofibromatosis or von Recklinghausen’s disease is known but exact pathophysiology has still not been clearly defined. [boneandspine.com]
Appropriate interpretation will improve understanding of the natural history and pathophysiology of neurofibromatosis type 1. [nature.com]
The pathophysiology of the resultant limb-length inequality may be related to the bowing. [emedicine.medscape.com]
Prevention
Moreover it does not always prevent ankle valgus [Figure 4]. To prevent all these, the rod must be kept till skeletal maturity. [doi.org]
At the end of the correction, a telescopic Fassier-Duval intramedullary nail was used prophylactically to prevent re-fracture. After 2 years of follow-up, X-ray images show bone-healing without any bowing. [ncbi.nlm.nih.gov]
poor protection against re-fracture, failure to correct limb length discrepancy, and deformities of leg and ankle simultaneously at time of primary surgery. 8 Vascularized fibular graft/ Nail The intramedullary rod offers good tibial alignment and prevents [slideplayer.com]
Created to prevent or stabilize fractures, or correct deformity of long bones whilst growth occurs. [pegamedical.com]